Idiopathic Pulmonary Fibrosis—Clinical presentation, outcome and baseline prognostic factors in a Portuguese cohort

Abstract

Introduction: Idiopathic Pulmonary Fibrosis (IPF) is the most common disease in the subgroup of idiopathic interstitial pneumonias. It is inevitably associated to a bad prognosis, although assuming a highly variable clinical course. Methods: Patients with IPF, observed at Interstitial Lung Diseases outpatient clinic of Centro Hospitalar de São João-Porto, Portugal, were identified and clinical, functional, radiological and bronchoalveolar lavage (BAL) parameters were reviewed. Their clinical course and survival were analyzed in order to identify prognostic factors. Results: Eighty-one patients were included, with a mean age at diagnosis of 63.8 years old. At diagnosis, the main functional abnormalities were restrictive physiology, reduced lung diffusion and exercise capacity impairment. Clinical course was mainly slowly progressive (72.3%). Ten patients (13.2%) had a rapid progression and 11 (14.5%) patients had an acute exacerbation during the course of the disease. IPF's rapid progression was associated to a higher functional impairment at diagnosis, namely in what is related with Functional Vital Capacity (FVC) and Total Lung Capacity (TLC). Median survival was 36 months. A significant difference in survival was observed among different types of clinical course-41 months for slow progressors and 9 months for rapid progressors. Lower levels of FVC, TLC, six-minute walk test (6MWT) distance and ଝ Please cite this article as: Pires FS, et al., Fibrose Pulmonar Idiopática-Apresentação clínica, evolução e factores prognósticos basais numa coorte portuguesa. Rev Port Pneumol. 2010.

Heterozygous TERT gene mutation associated with familial idiopathic pulmonary fibrosis

“…IPF is a devastating interstitial lung disorder of unknown etiology that typically leads to respiratory failure and death within 3–5 years after diagnosis [1,2,4]. It is usually a sporadic condition and rarely it is identified in families, those cases identified as Familial Pulmonary Fibrosis (FPF) [1].…”

“…Vertical transmission in families, suggests that FPF is inherited in an autosomal dominant fashion, although with incomplete penetrance (since not all of them develop disease in the presence of mutated genes). The age of onset is often younger than in sporadic IPF, approximately 55 years [2,[4], [5], [6]]. Several studies have compared the clinical, radiological, histological features and outcome of sporadic and familial IPF and they concluded that both diseases shared characteristics [[7], [8], [9]].…”

Management of Idiopathic Pulmonary Fibrosis Cases at a Tertiary Care Hospital

“…[3] A retrospective study suggested a potential benefit of treatment with azathioprine plus prednisone in a small case series. [9] A small randomized trial of corticosteroid versus corticosteroid and azathioprine showed a trend toward a survival benefit with combination therapy with corticosteroid and azathioprine. Pirfenidone is a pyridone compound with pleiotropic, antiinflammatory, antifibrotic, and antioxidant properties, with antagonism of TGF-b1 effects.…”

Idiopathic pulmonary fibrosis: accurate diagnosis and early treatment

“…For many years, combination therapy with corticosteroids and azathioprine, based on the mistaken context of persistent inflammation leading to fibrosis, and the subsequent addition of N-acetylcysteine, based on the idea that oxidative stress is a component of anomalous healing after alveolar epithelial damage with consequent extracellular matrix deposition and fibrosis progression, was used indiscriminately, which invariably led to the progression of the disease and to a mean survival rate of three years after diagnosis. (9,10) Subsequently, it was shown that this therapy did not provide any benefits in patients with IPF, rather causing significant adverse effects because the triple-therapy arm of the study had to be stopped prematurely due to a significant number of hospitalizations and deaths compared with the other two therapeutic arms of the study (i.e. N-acetylcysteine and placebo).…”